Sex Differences in Children and Young Adults With Bicuspid Aortic Valve Disease in First Two Decades of Life.

OBJECTIVE: To elucidate sex differences in valve morphology, disease phenotype, progression, and outcomes among children and young adults with bicuspid aortic valve (BAV). PATIENTS AND METHODS: This is a retrospective cohort study examining all children and young adults (aged ≤22 years) with isolated BAV diagnosed, by excluding patients with concomitant congenital heart defects or genetic syndromes, from January 1, 1990, through December 1, 2016, at Mayo Clinic in Rochester, Minnesota. RESULTS: Of 1010 patients with BAV, 558 had isolated BAV. Distributions of morphology were right-left in 65.8% (n=367), right-noncoronary in 34% (n=190), and left-noncoronary cusp fusion in 0.2% (n=1) of patients; with no sex differences. Male to female ratio was 3:1. At the first echocardiographic evaluation in the study, there were no sex differences in terms of frequency of aortic valve stenosis or regurgitation. However, males had significantly higher grades of aortic valve regurgitation at 17 years of age onward (P<.0001). Males had significantly larger mid-ascending aorta (P=.01) and sinus of Valsalva dimensions (z score; P=.0001) as compared with females, with a novel finding of peak aortic dimensions around 8 years of age. Males also had more than 2-fold higher risk for sinus of Valsalva dilation (z score >2) as compared with females (odds ratio, 2.3; 95% CI, 1.2 to 4.2; P=.01). There were no significant sex differences in the primary cardiac outcomes of interventions on aortic valve and/or aorta, aortic dissection, or death. CONCLUSION: In children and young adults with BAV, males have a higher grade of aortic regurgitation in late adolescence, significantly larger aortic dimensions, different patterns of aortic growth, and more frequent sinus of Valsalva dilation as compared with females. Overall, the rate of primary cardiac events is lower in young patients, with no significant sex differences.

Use of Electrocardiographic Screening to Clear Athletes for Return to Sports Following COVID-19 Infection.

OBJECTIVE: To quantify the occurrence rate of abnormal electrocardiographic (ECG) findings and symptoms following coronavirus disease 2019 (COVID-19) infection. PATIENTS AND METHODS: In this retrospective analysis, we studied adult patients (>18 years old) who were participating in collegiate athletics and previously tested positive for COVID-19 between August 1, 2020, and December 30, 2020. The athletes underwent general examinations and ECG screening prior to being medically cleared for a return to sports following their COVID-19 diagnosis. Predetermined predictors were grouped into categorical variables including (1) sex, (2) symptom severity, and (3) body mass index (normal vs overweight [≥24 kg/m2]). These variables were used to examine differences of abnormal rates that occurred between different predictor categories. RESULTS: Of the 170 athletes screened, 6 (3.5%) presented with abnormal ECG findings and were referred to cardiologists. We found no evidence that sex, symptom severity, and body mass index category were associated with a higher rate of abnormal ECG findings (all P>.05). Greater severity of COVID-19 symptoms was associated with a higher percentage of ST depression, T-wave inversion, ST-T changes, and the presence of fragmented QRS complex. Loss of smell, loss of taste, headache, and fatigue were the most prevalent symptoms, with 38.8% (66), 36.5% (62), 32.9% (56), and 25.3% (43), respectively, of the 170 athletes reporting each symptom. CONCLUSION: Preliminary findings indicate a low risk of myocardial injury secondary to COVID-19 infection, with less than 4% of the 170 patients in our study presenting with abnormal ECG findings and a total of 16 patients (9.4%) requiring referral to a cardiologist. Although viral myocarditis was not detected in any athlete referred for cardiological assessment, 2 patients experienced effusive viral pericarditis.

Hepatocellular carcinoma and the Fontan circulation: Clinical presentation and outcomes.

BACKGROUND: Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation. METHODS: A multicenter case series of Fontan patients with a diagnosis of HCC formed the basis of this study. The case series was extended by published cases and case reports. Clinical presentation, tumor characteristics, laboratory and hemodynamic findings as well as treatment types and outcomes, were described. RESULTS: Fifty-four Fontan patients (50% female) with a diagnosis of HCC were included. Mean age at HCC diagnosis was 30 ± 9.4 years and mean duration from Fontan surgery to HCC diagnosis was 21.6 ± 7.4 years. Median HCC size at the time of diagnosis was 4 cm with a range of 1 to 22 cm. The tumor was located in the right hepatic lobe in 65% of the patients. Fifty-one percent had liver cirrhosis at the time of HCC diagnosis. Fifty percent of the patients had no symptoms related to HCC and alpha-fetoprotein was normal in 26% of the cases. Twenty-six patients (48%) died during a median follow-up duration of 10.6 (range 1-50) months. CONCLUSIONS: HCC in Fontan patients occurs at a young age with a 1-year survival rate of only 50%. Meticulous liver surveillance is crucial to detect small tumors in the early stage.

Acute Pulmonary Embolism: Contemporary Approach to Diagnosis, Risk-Stratification, and Management.

Pulmonary embolism (PE) affects over 300,000 individuals each year in the United States and is associated with substantial morbidity and mortality. Improvements in the diagnostic performance and availability of computed tomographic pulmonary angiography and D-dimer testing have facilitated the evaluation of patients with suspected PE. High clinical suspicion is required in those with risk factors and/or those that manifest signs or symptoms of venous thromboembolic disease, with validated clinical risk scores such as the Wells and modified Wells score or the PE rule-out criteria helpful in estimating the likelihood for PE. For those with confirmed PE, patients should be categorized and triaged according to the presence or absence of shock or hypotension. Normotensive patients can be further risk-stratified using validated prognostic risk scores, as well as by using imaging and cardiac biomarkers, with those having either signs of right ventricular dysfunction on imaging studies and/or abnormal cardiac biomarkers categorized as being at intermediate-risk and requiring close monitoring and hospital admission. Early discharge and/or home therapy are possible in those that do not manifest any high-risk features. The initial treatment for most patients that are stable consists of anticoagulation, with advanced therapies such as thrombolysis, catheter-based therapies, or surgical embolectomy deferred for those at high risk. Given the heterogeneous presentations of PE and various management strategies available, the development of multidisciplinary PE response teams has emerged to help facilitate decision-making in these patients.

Vasopressor magnitude predicts poor outcome in adults with congenital heart disease after cardiac surgery.

BACKGROUND: High levels of vasoactive inotrope support (VIS) after congenital heart surgery are predictive of morbidity in pediatric patients. We sought to discern if this relationship applies to adults with congenital heart disease (ACHD). METHODS: We retrospectively studied adult patients (≥18 years old) admitted to the intensive care unit after cardiac surgery for congenital heart disease from 2002 to 2013 at Mayo Clinic. Vasoactive medication dose values within 96 hours of admission were examined to determine the relationship between VIS score and poor outcome of early mortality, early morbidity, or complication related morbidity. RESULTS: Overall, 1040 ACHD patients had cardiac surgery during the study time frame; 243 (23.4%) met study inclusion criteria. Sixty-two patients (25%), experienced composite poor outcome [including eight deaths within 90 days of hospital discharge (3%)]. Thirty-eight patients (15%) endured complication related early morbidity. The maximum VIS (maxVIS) score area under the curve was 0.92 (95% CI: 0.86-0.98) for in-hospital mortality; and 0.82 (95% CI: 0.76-0.89) for combined poor clinical outcome. On univariate analysis, maxVIS score ≥3 was predictive of composite adverse outcome (OR: 14.2, 95% CI: 7.2-28.2; P < 0.001), prolonged ICU LOS ICU LOS (OR: 19.2; 95% CI: 8.7-42.1; P < 0.0001), prolonged mechanical ventilation (OR: 13.6; 95% CI: 4.4-41.8; P < 0.0001) and complication related morbidity (OR: 7.3; 95% CI: 3.4-15.5; P < 0.0001). CONCLUSIONS: MaxVIS score strongly predicted adverse outcomes and can be used as a risk prediction tool to facilitate early intervention that may improve outcome and assist with clinical decision making for ACHD patients after cardiac surgery.

Practical Implementation of Failure Mode and Effects Analysis for Extracorporeal Membrane Oxygenation Activation.

Extracorporeal membrane oxygenation (ECMO) is used to treat severe hypoxemic respiratory failure and as a rescue therapy for patients with cardiopulmonary arrest within a narrow window of time. A failure modes and effects analysis (FMEA) was conducted to analyze the clinical and operational processes leading to delays in initiating ECMO. FMEA determined these highest-risk failure modes that were contributing to process failure: (1) ECMO candidacy not determined in time, (2) no or incomplete evaluation for ECMO prior to consult or arrest, (3) ECMO team not immediately available, and (4) cannulation not completed in time. When implemented collectively, a total of 4 interventions addressed more than 95% of the system failures. These interventions were (1) ECMO response pager held by a team required for decision, (2) distribution of institutionally defined inclusion/exclusion criteria, (3) educational training for clinicians consulting the ECMO team, and (4) establishment of a mobile ECMO insertion cart.

Characteristic Morphologies of the Bicuspid Aortic Valve in Patients with Genetic Syndromes.

BACKGROUND: In patients with bicuspid aortic valve (BAV), complications including progressive aortic stenosis and aortic dilatation develop over time. The morphology of cusp fusion is one of the determinants of the type and severity of these complications. We present the association of morphology of cusp fusion in BAV patients with distinctive genetic syndromes. METHODS: The Mayo Clinic echocardiography database was retrospectively reviewed to identify patients (age ≤ 22 years) diagnosed with BAV from 1990 to 2016. Cusp fusion morphology was determined from the echocardiographic studies, while coexisting cardiac defects and genetic syndromes were determined from chart review. RESULTS: A total of 1,037 patients with BAV were identified: 550 (53%) had an isolated BAV, 299 (29%) had BAV and a coexisting congenital heart defect, and 188 (18%) had BAV and a coexisting genetic syndrome or disorder. There were no differences in distribution of morphology across the three groups. However, right-noncoronary (RN) cusp fusion was the predominant morphology associated with Down syndrome (P = .002) and right-left (RL) cusp fusion was the predominant morphology associated with Turner syndrome (P = .02), DiGeorge syndrome (P = .02), and Shone syndrome (P = .0007), when compared with valve morphology in patients with isolated BAV. Isolated BAV patients with RN cusp fusion had larger ascending aorta diameter (P = .001) and higher number of patients with ≥ moderate aortic regurgitation (P = .02), while those with RL cusp fusion had larger sinus of Valsalva diameter (P = .0006). CONCLUSIONS: Morphological subtypes of BAV are associated with different genetic syndromes, suggesting distinct perturbations of developmental pathways in aortic valve malformation.

New-Onset Heart Failure and Mortality in Hospital Survivors of Sepsis-Related Left Ventricular Dysfunction.

BACKGROUND: The association between new-onset left ventricular (LV) dysfunction during sepsis with long-term heart failure outcomes is lesser understood. METHODS: Retrospective cohort study of all adult patients with severe sepsis and septic shock between 2007 and 2014 who underwent echocardiography within 72 h of admission to the intensive care unit. Patients with prior heart failure, LV dysfunction, and structural heart disease were excluded. LV systolic dysfunction was defined as LV ejection fraction <50% and LV diastolic dysfunction as ≥grade II. Primary composite outcome included new hospitalization for acute decompensated heart failure and all-cause mortality at 2-year follow-up. Secondary outcomes included persistent LV dysfunction, and hospital mortality and length of stay. RESULTS: During this 8-year period, 434 patients with 206 (48%) patients having LV dysfunction were included. The two groups had similar baseline characteristics, but those with LV dysfunction had worse function as demonstrated by worse LV ejection fraction, cardiac index, and LV diastolic dysfunction. In the 331 hospital survivors, new-onset acute decompensated heart failure hospitalization did not differ between the two cohorts (15% vs. 11%). The primary composite outcome was comparable at 2-year follow-up between the groups with and without LV dysfunction (P = 0.24). Persistent LV dysfunction was noted in 28% hospital survivors on follow-up echocardiography. Other secondary outcomes were similar between the two groups. CONCLUSIONS: In patients with severe sepsis and septic shock, the presence of new-onset LV dysfunction did not increase the risk of long-term adverse heart failure outcomes.

Role of Admission Troponin-T and Serial Troponin-T Testing in Predicting Outcomes in Severe Sepsis and Septic Shock.

BACKGROUND: Troponin-T elevation is seen commonly in sepsis and septic shock patients admitted to the intensive care unit. We sought to evaluate the role of admission and serial troponin-T testing in the prognostication of these patients. METHODS AND RESULTS: This was a retrospective cohort study from 2007 to 2014 on patients admitted to the intensive care units at the Mayo Clinic with severe sepsis and septic shock. Elevated admission troponin-T and significant delta troponin-T were defined as ≥0.01 ng/mL and ≥0.03 ng/mL in 3 hours, respectively. The primary outcome was in-hospital mortality. Secondary outcomes included 1-year mortality and lengths of stay. During this 8-year period, 944 patients met the inclusion criteria with 845 (90%) having an admission troponin-T ≥0.01 ng/mL. Serial troponin-T values were available in 732 (78%) patients. Elevated admission troponin-T was associated with older age, higher baseline comorbidity, and severity of illness, whereas significant delta troponin-T was associated with higher severity of illness. Admission log10 troponin-T was associated with unadjusted in-hospital (odds ratio 1.6; P=0.003) and 1-year mortality (odds ratio 1.3; P=0.04), but did not correlate with length of stay. Elevated delta troponin-T and log10 delta troponin-T were not significantly associated with any of the primary or secondary outcomes. Admission log10 troponin-T remained an independent predictor of in-hospital mortality (odds ratio 1.4; P=0.04) and 1-year survival (hazard ratio 1.3; P=0.008). CONCLUSIONS: In patients with sepsis and septic shock, elevated admission troponin-T was associated with higher short- and long-term mortality. Routine serial troponin-T testing did not add incremental prognostic value in these patients.

Benign nodules in post-Fontan livers can show imaging features considered diagnostic for hepatocellular carcinoma.

PURPOSE: To describe the imaging appearance of hyperenhancing nodules arising in post-Fontan patients and to identify specific features best correlated with malignancy. METHODS: Hyperenhancing hepatic nodules visible on CT and/or MRI in post-Fontan patients were identified retrospectively and reviewed by subspecialty radiologists. Nodules with characteristic imaging findings of focal nodular hyperplasia (FNH) were defined as typical, the remainder were defined as atypical, described in detail according to LIRADS criteria, and length of stability over time was recorded. Clinical data, alpha fetoprotein levels (AFP), central venous pressures (CVP), and histopathology were recorded. RESULTS: 245 hyperenhancing nodules (215 typical, 30 atypical) were evaluated in 30 patients. Twenty-nine atypical nodules showed washout (portal phase in 6, delayed phase in 29), 0 showed pseudocapsule, 1 showed threshold growth, 1 showed tumor in vein, and 5 showed ancillary features favoring malignancy. Pathology confirmed hepatocellular carcinoma (HCC) in 3 atypical nodules and FNH-like histology in 3 atypical and 4 typical nodules. 2 atypical nodules were present in a patient with clinical diagnosis of HCC. 20 nodules (7 typical, 13 atypical due to washout) were studied with hepatobiliary contrast agent and all showed homogenous hepatobiliary phase retention. Atypical nodules were significantly more likely to be HCC than biopsy-proven FNH-like or stable ≥24 months when showing portal phase washout (P < 0.001), mosaic architecture (P = 0.020) or in the presence of cirrhosis (P = 0.004) or elevated AFP (P = 0.004). Atypical nodules that were HCC had higher median CVP than those that were FNH-like (19, range 16-27 vs. 13, range 12-16 mmHg, P = 0.0003), there was not a significant difference based on median patient age (HCC 30, range 10-41 vs. FNH-like 40 range 10-41, P = 0.244). CONCLUSIONS: Benign hyperenhancing masses in Fontan patients may demonstrate washout and be mistaken for HCC by imaging criteria. Portal phase washout, mosaic architecture, elevated AFP and higher CVP were associated with HCC in the atypical nodules found in this population.

Bicuspid and Unicuspid Aortic Valve: Fate of Moderate/Severe Mixed Aortic Valve Disease.

OBJECTIVES: There is a paucity of data about mixed aortic valve disease (MAVD) in patients with bicuspid/unicuspid aortic valve (BAV). This study sought to describe the outcomes of patients with moderate/severe MAVD. METHODS: We queried our database for patients with BAV and moderate/severe MAVD seen between 1994 and 2013. We excluded patients with baseline New York Heart Association (NYHA) III/IV symptoms, left ventricular ejection fraction <50%, aortic dimension >50 mm, and significant disease of other valves. The purpose of the study was to determine the freedom from NYHA III/IV symptoms and aortic valve replacement (AVR). RESULTS: We identified 138 patients with moderate/severe MAVD; mean age was 51 ± 12 years; 112 (81%) were males; and follow-up was 8.5 ± 4 years. Ninety-two patients (67%) underwent AVR within 3.7 ± 2.5 years. Mechanical prostheses were implanted in 73 patients (79%); 22 patients (26%) and 36 patients (39%) had concomitant coronary artery bypass graft and aorta replacement during AVR respectively. There were no surgical deaths. Freedom from AVR was 84%, 51%, and 20% at 1, 5 and 10 years respectively. Predictors of AVR were age at presentation (hazard ratio [HR] 5.22; confidence interval [CI] 3.10 to 6.64) for every decade increase in age; and having severe stenosis or regurgitation at the time of presentation (HR 1.32; CI 1.05 to 3.16). CONCLUSIONS: Age and disease severity should be incorporated in the risk assessment of BAV patients with MAVD, and patients with both risk factors should be monitored closely.

Prevalence and outcome of thrombotic and embolic complications in adults after Fontan operation.

There are limited studies of thrombotic and embolic complications (TEC) in the adult Fontan population. The purpose of the study was to determine the prevalence, risk factors, and outcomes of TECs in this population. METHODS: Retrospective review of adults with a previous Fontan operation, with follow-up at Mayo Clinic, 1994-2014. Systemic TEC was defined as intracardiac thrombus, ischemic stroke, or systemic arterial embolus. Nonsystemic TEC was defined as Fontan conduit/right atrial thrombus or pulmonary embolus. RESULTS: We identified 387 patients with a mean (SD) age of 28 (7) years and a mean follow-up of 8 (2) years. An atriopulmonary connection (APC) was done for 286 patients (74%). Atrial arrhythmias were present in 278 (72%). There were 121 TECs (systemic n=36, nonsystemic n=85) in 98 patients (25%). Risk factors for systemic TEC were atrial arrhythmia (hazard ratio 2.28, P=.001) and APC (hazard ratio 1.98, P=.02); nonsystemic TEC also had similar risk factors. All 98 patients received warfarin. Warfarin was discontinued in 10 of 98 because of bleeding, and 8 of these 10 subsequently had a second TEC. Among the 82 patients who had follow-up imaging, 16 (20%) had resolution of thrombus. In total, 24 of 98 patients had a second TEC, most of whom had inadequate anticoagulation. CONCLUSIONS: Thrombotic and embolic complication was not uncommon; risk factors for TEC were APC and atrial arrhythmias. Most patients were treated successfully with warfarin alone. A second TEC occurred in most patients whose anticoagulation was discontinued because of bleeding events.

Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects.

BACKGROUND: Bicuspid aortic valve (BAV) occurs both as an isolated cardiac lesion and in association with congenital heart defects (CHD). Their aim was to identify the incidence and morphology of BAV in patients with coexisting CHD and compare their disease progression to patients with isolated BAV. METHODS: The Mayo Clinic echocardiography database was retrospectively analyzed to identify pediatric and young adult patients (≤22 years) who were diagnosed with BAV from 1990 to 2015. The morphology of BAV was determined from the echocardiographic studies before any intervention. RESULTS: Overall, 1010 patients with BAV were identified, 619 (61%) with isolated BAV and 391 (39%) with BAV and coexisting CHD. The incidence of BAV was highest in patients with coarctation of the aorta (36%) and interrupted aortic arch (36%). In comparison to patients with isolated BAV, patients with BAV and left-sided obstructive lesions more frequently had right-left cusp fusion (P = .0001). BAV in patients with right-sided obstructive lesions was rare, but they more frequently had right-noncoronary or left-noncoronary cusp fusion (P = .01). No significant progression of aortic stenosis or regurgitation was observed in patients with BAV and coexisting CHD; however in patients with isolated BAV the severity of aortic regurgitation increased with age. In patients with isolated BAV, the ascending aorta diameter (z-score) increased with age, peaked around 8-9 years of age, and was larger in comparison to patients with BAV and coexisting CHD. The sinus of Valsalva diameter (z-score) in patients with BAV and ventricular septal defect was larger than isolated BAV patients after 18 years (P < .04). CONCLUSIONS: The morphology of BAV, the pattern and progression of aortic dilatation, and the severity of aortic valve disease vary in pediatric and young adult patients with BAV and coexisting CHD. However, there was no significant BAV disease progression when associated with these CHD.

Intravascular and hybrid intraoperative stent placement for baffle obstruction in transposition of the great arteries after atrial switch.

OBJECTIVES: To report our experience with intravascular and hybrid intra-operative stent placement for baffle obstruction in patients with complete transposition of the great arteries (TGA) after the atrial switch (Mustard/Senning) operation. BACKGROUND: Venous baffle obstruction is a challenging complication after atrial switch operation in patients with TGA. Traditional treatment options include intravascular stenting or surgery. METHODS: A retrospective analysis of Mayo Clinic's electronic medical record was completed to identify consecutive pediatric and adult patients with TGA after atrial switch who underwent baffle stent implantation from 1994 to 2015. RESULTS: Overall, 64 patients were referred for cardiac catheterization, in whom 47 (73%) were noted to have hemodynamic and angiographic evidence of baffle obstruction. A total of 20 patients mean age 33 (range: 8-46) years old underwent stent implantation of baffle stenosis at a mean of 33 (range: 7.5-45) years after initial atrial switch operation (Mustard, n = 19; Senning, n = 1). Overall, 27 baffles were stented in 20 patients via the following approaches: intravascular (17); hybrid surgical (3); staged intravascular & hybrid (2). Sites of stent placement were: superior vena cava (SVC) (13); inferior vena cava (IVC) (9); pulmonary venous baffle (5). Three patients had stent placement in the SVC and IVC baffles during the same procedure. Procedural adverse events occurred in 2/22 cases (9%) including creation of unintentional baffle leak (n = 1) and stent migration (n = 1). There was no procedure-related mortality. At follow-up (median 2, range 0.02-10 years), significantly improved NYHA class and mean Doppler baffle gradient were demonstrated (P < 0.05). Mild baffle re-stenosis (mean Doppler gradient; 2-3 mmHg) occurred in two patients who have not required re-intervention. Trivial baffle leak was noted in four patients. Baffle re-intervention was only occurred in one patient. One patient with pre-procedural Class IV symptoms died 3.5 months after stent implantation. CONCLUSIONS: This study is the largest reported experience of intravascular and intra-operative hybrid stent placement for patients with TGA after atrial switch. Transcatheter and minimally invasive hybrid intraoperative surgical stent placement are an effective strategy for relief of systemic and pulmonary venous baffle obstruction. © 2016 Wiley Periodicals, Inc.

Thrombotic and Embolic Complications Associated With Atrial Arrhythmia After Fontan Operation: Role of Prophylactic Therapy.

BACKGROUND: There are limited data about the risk of thrombotic and embolic complication (TEC) in adults with atrial arrhythmia after Fontan operation. OBJECTIVES: This study sought to determine the risk of TEC in this population and the role of anticoagulation therapy in TEC prevention. METHODS: This was a retrospective review of adults with atrial arrhythmia after Fontan operation who were evaluated at the Mayo Clinic between 1994 to 2014. TEC was classified into 2 groups: systemic TEC, defined as intracardiac thrombus, ischemic stroke, or systemic arterial embolus; and nonsystemic TEC, defined as Fontan conduit/right atrial thrombus or pulmonary embolus. Patients were divided into 3 groups: anticoagulation, antiplatelet, and no therapy cohorts. RESULTS: We followed 278 patients, mean age 31 ± 9 years, for 88 ± 14 months (1,464 patient-years). Patient groups included antiplatelet (n = 181), anticoagulation (n = 91), and no therapy (n = 6). There were 97 TEC in 81 patients (29%); 32 were systemic, yielding an event rate of 2.1 systemic TEC per 100 patient-years, and 65 were nonsystemic TEC, yielding an event rate of 4.4 nonsystemic TEC per 100 patient-years. Prevalence of TEC was 18% and 55% at 5 and 10 years, respectively. Atriopulmonary connection was a risk factor for TEC (hazard ratio: 2.31; 95% confidence interval: 1.61 to 4.64), and TEC were associated with higher risk of death and hospitalization (p < 0.0001). Anticoagulation was protective against TEC and resulted in a reduction of TEC risk by 2.5 TEC per 100 patient-years. Anticoagulation was also associated with lower risk of death and hospitalization (p = 0.02). Bleeding complications occurred in 21 (7%) patients and were similar in all groups. CONCLUSIONS: Anticoagulation was associated with lower TEC rate and lower risk of death and hospitalization, without a significant increase in bleeding risk. Perhaps anticoagulation should be the preferred preventive strategy.

When is the right time for Fontan conversion? The role of cardiopulmonary exercise test.

BACKGROUND: To determine if Fontan conversion (FC) resulted in improvement in exercise capacity (EC), and to determine the role of cardiopulmonary exercise test (CPET) in risk stratification of patients undergoing FC. METHODS: A retrospective review of patients who underwent CPET prior to FC at Mayo Clinic from 1994 to 2014. The patients who also underwent post-operative CPET were selected for the analysis of improvement in EC defined as 10% increase in baseline peak oxygen consumption (VO2). RESULTS: 75 patients CPET prior to FC; mean age 24±6years; 44 males (59%); and 51 (68%) were in NYHA III/IV prior to FC. Pre-operative peak VO2 was 15.5±3.4ml/kg/min. A comparison of pre- and post-FC CPET data was performed using 42 patients (56%) that underwent CPET after FC. Improvement in EC occurred in 18 of 42 patients (43%). Baseline peak VO2 >14ml/kg/min was associated with improved EC (hazard ratio [HR] 1.85; P=.02). Improvement in New York Heart Association (NYHA) class occurred in 12 (67%) patients with improved EC vs 2 (8%) without improved EC. Improvement in NYHA class was more likely to occur in patients with improved EC compared to those without improvement EC (odds ratio 4.11, P=.01). There were 10 (13%) perioperative deaths, and baseline peak VO2 ≤14ml/kg/min was predictive of perioperative mortality (HR 3.74; P<.001). CONCLUSIONS: Baseline peak VO2 was predictive of perioperative survival, and improvement in EC. Performance on CPET in failing Fontan patients might be a useful clinical parameter in determining appropriate timing of FC.

Imaging Findings of Congestive Hepatopathy.

Congestive hepatopathy (CH) refers to hepatic abnormalities that result from passive hepatic venous congestion. Prolonged exposure to elevated hepatic venous pressure may lead to liver fibrosis and cirrhosis. Liver dysfunction and corresponding clinical signs and symptoms typically manifest late in the disease process. Recognition of CH at imaging is critical because advanced liver fibrosis may develop before the condition is suspected clinically. Characteristic findings of CH on conventional images include dilatation of the inferior vena cava and hepatic veins; retrograde hepatic venous opacification during the early bolus phase of intravenous contrast material injection; and a predominantly peripheral heterogeneous pattern of hepatic enhancement due to stagnant blood flow. Extensive fibrosis can be seen in chronic or severe cases. Hyperenhancing regenerative nodules that may retain hepatobiliary contrast agents are often present. Magnetic resonance (MR) elastography can show elevated liver stiffness and may be useful in evaluation of fibrosis in CH because it can be incorporated easily into routine cardiac MR imaging. Preliminary experience with MR elastography suggests its future use in initial evaluation of patients suspected of having CH, for monitoring of disease, and for assessment after therapy. To facilitate appropriate workup and treatment, radiologists should be familiar with findings suggestive of CH at radiography, ultrasonography, computed tomography, MR imaging, and MR elastography. In addition, knowledge of underlying pathophysiology, comparative histologic abnormalities, and extrahepatic manifestations is useful to avoid diagnostic pitfalls and suggest appropriate additional diagnostic testing. (©)RSNA, 2016.

Cone Reconstruction of Atypical Ebstein Anomaly Associated with Right Ventricular Apical Hypoplasia.

Cone reconstruction for tricuspid valve repair has revolutionized the surgical treatment of Ebstein anomaly. We present the case of a 58-year-old woman with atypical Ebstein anomaly and right ventricular apical hypoplasia who was spared from palliative shunt physiology by our use of cone reconstruction. Compared with other techniques, cone reconstruction of the tricuspid valve more closely replicates normal valvular anatomy and function. This surgical procedure can be applied to many anatomic variations of Ebstein anomaly, as in our patient's apparently unique instance of atypical Ebstein anomaly with right ventricular apical hypoplasia.

Improved Ventricular Function after TEE-guided Cardioversion of Atrial Arrhythmias in Patients after the Fontan Operation.

OBJECTIVE: Atrial tachyarrhythmias frequently develop after the Fontan operation. Patients with Fontan physiology rely on atrial contribution to cardiac output, and thus control of atrial arrhythmias is important. Outcomes after cardioversion in patients after Fontan have not been reported. We sought to determine if cardioversion results in improved echocardiographic parameters or clinical symptomatology; and, discern risk factors for arrhythmia recurrence. DESIGN: We retrospectively analyzed the Mayo Clinic echocardiographic database to capture patients after the Fontan operation who underwent transesophageal echocardiography-guided electrical cardioversion from 2000-2015. Clinical and echocardiographic data were collected and compared at baseline and follow-up. RESULTS: Eight hundred ninety patients with prior Fontan operation underwent echocardiographic evaluation; 341 (38%) developed atrial arrhythmias. Thirty-six patients [20 males, median age 29 (12-51)] underwent transesophageal echocardiography-guided cardioversion of atrial arrhythmias [atrial flutter/intraatrial reentrant tachycardia (75%); atrial fibrillation (25%)]. At follow-up, improvements were noted in ejection fraction by 10% (P < .0001); atrioventricular valve regurgitation grade (39%) (P = .002); New York Heart Association (NYHA) class (61%) (P < .001); and resolution of spontaneous echo contrast in the Fontan circuit (65%) (P < .01). No embolic events occurred following cardioversion. Eighteen patients (50%) developed recurrent atrial arrhythmias at 15 (3-36) months after cardioversion. Five-year freedom from arrhythmia recurrence was 61%. Significant univariate predictors of arrhythmia recurrence were atrial flutter/intraatrial reentrant tachycardia (HR = 4.3, P = .02); NYHA ≥ II (HR = 4.1, P = .03); systemic right ventricle (HR = 5.2; P = .02); and ejection fraction ≤ 40% (HR = 2.8; P = .04). On multivariate analysis, only systemic right ventricle (HR = 3.7; P = .02) remained an independent predictor of arrhythmia recurrence. CONCLUSION: After the Fontan operation, cardioversion of atrial arrhythmias improves ventricular function, atrioventricular valve regurgitation grade, and NYHA class. Arrhythmia recurrence was common and patients with atrial flutter/intraatrial reentrant tachycardia, systemic right ventricle, or reduced ventricular function may be at risk of arrhythmia recurrence. Further studies are required to identify additional risk factors and protective factors for arrhythmia recurrence.